Contrast-induced encephalopathy (CIE) is a rare adverse event associated with the intravascular administration of iodine-containing contrast media. Neuroendovascular interventions and cardiac catheterization are the most common procedures leading to CIE. Patient comorbidities like hypertension and renal failure are common. CIE manifests due to acute neurotoxicity, with clinical symptoms that can mimic acute ischemic stroke or acute encephalopathy, including hemiparesis, cortical blindness, and seizures. The understanding of CIE is incomplete, with its pathophysiology likely involving a transient breakdown of the blood-brain barrier, leading to neurotoxicity and imaging findings such as contrast staining and vasogenic edema. Differentiating CIE from other acute conditions that require immediate treatment is crucial, as CIE is usually self-limiting with a generally favorable outcome. Still, rare cases with fatal or prolonged neurological deficit exist. The prevalence of CIE is rising due to the increased frequency of endovascular procedures, e.g., for acute coronary disease and stroke. Lack of knowledge of this rare complication leads to misdiagnoses, unnecessary diagnostic tests, and wrong or unnecessary treatments.